JK SCIENCE
Vol. 20 No. 1, Jan-March 2018 www.jkscience.org 49
CASE REPORT
Enlarged Vestibular Aqueduct Syndrome (Evas) -A Rare Disorder In Children
Amandeep Arora, Monika Gupta
EVAS is a rare identity in children. Male to female
ratio is found to be 1:1.5 (1). The term enlarged vestibular
aqueduct is used when a vestibular aqueduct is larger
than normal. Some studies suggest that the vestibular
aqueduct is enlarged if it is more than 1.5mm in diameter
while other define EVAS as being more than 4mm in
size (2). It is a MRI finding, so MRI is considered to be
the most appropriate test (2,3).
Case Report:
Two sisters at the age of 6yr and 5yr brought with
chief complaints of not able to hear and speak by birth.
There is no any other complaints of vertigo, unsteadiness,
decreased vision. No any dysmorphic features and no
history of trauma present. On general examination both
the girls are active normal in appearance ,no any
dysmorphic features are present. Systemic examination
are within normal limits. BERA was done which shows
moderate to severe SNHL present. From the clinical
standpoint, SNHL is the unique presentation in EVAS
among children (4) OAE-Absent in both ears.
MRI pictures of both sisters shows enlarged bilateral
vestibular aqueduct and endolymphatic sac. Younger sister
Introduction
Abstract EVAS is considered to be rare disorder and its true prevalence is not known. MRI is considered to be themost appropriate diagnostic test. We report 5yr and 6yr old sisters who came to our OPD with complaintsof not able to hear and not able to speak by birth.
Key WordsEnlarged vestibular aqueduct syndrome(EVAS)
From the Department of Pediatrics & Pathology, Adesh Institute of Medical Science & Research Institute Bathinda, Punjab, IndiaCorrespondence to : Dr.Monika Gupta, 191/62, Mashakganj, Bagh Sher Jung, Lucknow
Fig 1. MRI pictures shows enlarged bilateral vestibularaqueduct and endolymphatic sac. Younger sister 5yr havingAP diameter of aqueduct is 3.3 mm on right side and 2.9 mmon left side
JK SCIENCE
50 www.jkscience.org Vol. 20 No. 1, Jan.-March 2018
5yr having AP diameter of aqueduct is 3.3 mm on right
side and 2.9 mm on left side( Fig 1) Older sister 6yr
having AP diameter of aqueduct 2.9 mm on right side
and 4.6 mm on left side.(Fig 2)
Discusion
The present case is relevant because of the rarity of
this disease in the children. It is a hearing loss that usually
brings EVAS to the attention of a pediatrician. Such loss
can be sensorineural, conductive or both. Clinical signs
vary from hearing loss to vestibular symptoms like
spinning vertigo, unsteadiness, feeling of vague instability.
In our case both sisters are not able to hear and speak
by birth otherwise all general and systemic examination
is within normal limits.
In addition to complete medical history and
physical examination, to diagnose a case of EVAS, requires
hearing testing and radiological evaluation. In children
with SNHL incidence of radiological abnormalities has
been reported to be as high as 39%. (5).
On Audiological testing bilateral sensorineural hearing
loss was present.
1. MaddenC, Halsted AB, Corning GJ, et al. Enlarged
Vestibular Aqueduct Syndrome in the Pediatric
Population.Otology& Eurotology 2003;24: 625-32.
2. Turski PA, Seidenwurm DJ, David PC, et al. ACR
[American College of Radiology] Appropriatness criteria:
vertigo and hearing loss. Available at: www.guideline.gov/
summary /summary.aspx?doc_id=9602&nbr=005123
3. US National Library of medicine. Nonsyndromic
Deafness,[Cited in Dec 14,2009],Available at: http://
ghr.nlm.nih.gov/condition= nonsyndromicdeafness.
4. Mafong DD, Shin E, Lalwani A K. Use of laboratory
evaluation and radiologic imaging in the diagnostic evaluation
of children with sensorineural hearing loss. Laryngoscope
2002;112:1-7.
5. Vijayasekaran S, Halsted MJ, Boston M, et al. When Is the
Vestibular Aqueduct Enlarged? A Statistical Analysis of the
Normative Distribution of Vestibular Aqueduct Size. AJNR
2007 ;28: 1133-38.
6. Miyamoto RT, Bichey BG, Wynne MK, Kirk KI. Cochlear
implantation with large vestibular aqueduct syndrome.
Laryngoscope 2002; 112:1178-82.
References
There was no nystagmus and no other vestibular
symptoms present. So vestibular system is considered to
be normal.
For radiological assessment MRI was done which
shows enlarged bilateral vestibular aqueduct and
endolymphatic sac in both the sisters.
In such cases cochlear implant has significantly
improved hearing (6).
Fig 2. MRI pictures of both shows enlarged bilateral vesti-bular aqueduct and endolymphatic sac. Older sister 6yrhaving AP diameter of aqueduct 2.9 mm on right side and4.6 mm on left side